PUO with a H/O RECURRENT SEIZURES IN A 18yr OLD MALE
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A 18 year old male who is a student and a resident of Hyderabad presented with the chief complaints of
Generalised body pains since 4 days.
Cough without expectoration since 1 day.
History of presenting illness-
h/o rt ear discharge with pain(on and off) since 1 year.— 2-3 episodes/yr lasting for 6-7 days. Last episode - 2months back.
Discharge- mucous, moderate, intermittent, non foul smelling, non blood stained. Treated for the same every time.
The patient came to the OPD with main C/O backache when he developed high grade fever which was sudden in onset and varied between a range of 100-104 without touching the baseline. It was a/w chills and rigor. (Since 4 days)
Severe back pain since 4 days. It is so severe that he cries out due to it. He needs support to get up.
He has had episodes of vomiting on day 2 and day 4 of symptoms.
The vomitus contained food particles, it was non bilious and non projectile.
He has taken Ayurvedic medication on day 3 of fever for the same.
He also complains of pain along SCM. Which aggrevated in touch.
He has pain while opening of jaw but there is no pain while closing it.
Personal history:
Appetite : decreased for the past 4 days
Diet : mixed
Bowel and bladder : normal
Urine is frothy.
Sleep : adequate
Addictions : -
Family history:
Father and sister has had an episode of febrile seizure at around 5yrs of age, lasting for 2mins, no treatment taken. No further medication was taken.
General examination:
The patient was examined after a well informed consent in a well lit room.
The patient was conscious, coherent and cooperative. He was well oriented to time, place and person.
He is moderately built and nourished.
No signs of pallor, icterus, cyanosis, clubbing, generalised lymphadenopathy and pedal edema were seen.
Vitals-
Temperature : 100°C
BP : 130/80mm hg
Rerpiratory rate : 18cpm
Pulse rate : 100bpm
Systemic examination:
CNS : reflexes- normal
Tone - normal power- 5 in all four limbs
Sensory system - intact
Kernigs - terminal rigidity- + (on day 1)
Neck rigidity- present on day 1
Brudinzkis- absent
Neck rigidity- resolved by day 2
Kernigs- terminal rigidity - absent by day 2
CVS: S1 and S2 are heard. No murmurs we’re heard.
Respiratory system: BAE+ no adventitious sounds were heard
Per abdominal examination: soft , non-tender and no organomegaly was noted.
INVESTIGATIONS-
DIAGNOSIS-
PUO WITH RECURRENT FEBRILE SEIZURES
Diagnosed on day 3- ?PERIODIC FEVER SYNDROME (?PFAPA-periodic fever, aphthous stomatitis, pharyngitis, adenitis) WITH RECURRENT ENCEPHALITIS AND IGA NEPHROPATHY ?
TREATMENT-
DAY 1-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
INJ.OPTINEURON 1AMP IN 100 ML NS
INJ.CEFTRIAXONE IG IV/BD
T.CEVIPIL 500MG PO/BD
TEMP. CHARTING
GRBS 12TH HRLY
ENT REFERRAL- done in view of previous h/o rt ear discharge with pain(on and off) since 1 year. 2-3 episodes/yr lasting for 6-7 days. Last episode - 2months back.
Discharge- mucous, moderate, intermittent, non foul smelling, non blood stained.
O/E nose,ear - NORMAL, NO INTERVENTION NEEDED.
Daily routine of the patient
The patient wakes up everyday at 7:00am. He has breakfast and gets ready to go to college. He travels to his college by bus. He attends his college from 9:00am to 3:00pm. He comes back home by bus. From 4:00pm to 6:00pm he freshens up and takes rest. At 6:00pm he goes to attend Tutions for an hour after which he comes back home and completes his homework. He has dinner at 9:00pm and sleeps at 10:30pm .
The frequent admissions and illness of the patient is subjecting him to mental and academic stress. Mentally, The patient has become more aggressive, stubborn day by day. Academically he has had a lot of halts. The tuition teacher says his memory is very much hampered. The parents do not know if it’s due to the frequent illness or not. Also, the parents say he tends to get ill during times of stress like exams or something as such.
References-
https://www.rileychildrens.org/health-info/periodic-fever-syndrome
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2394724/
https://onlinelibrary.wiley.com/doi/full/10.1111/ped.12077
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505547/ - ****
Inference-
"Periodic fever, Aphthous-stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
What is Periodic fever, Aphthous-stomatitis, Pharyngitis, Adenitis (PFAPA) syndrome?
This syndrome includes recurrent episodes of fever with aphthous-stomatitis (mouth sores), pharyngitis (sore throat with redness and sometimes a throat that has a white covering – exudate - like that’s seen in a throat with streptococcal infection). PFAPA affects children in early childhood, usually starting at age 2 to 4 years. Episodes usually decrease in frequency and resolve after the age of 10 years. This disease was recognized for the first time in 1987 and was called Marshall’s.
How common is PFAPA syndrome?
The frequency of PFAPA is not known, but the disease appears to be more common than generally appreciated.
What causes PFAPA syndrome?
The answer to this question is not yet known. No gene defect has been found in PFAPA, although in some cases more than one family member has the disease. No infectious cause has been found in PFAPA, thus it is not a contagious disease. It is clear that the inflammatory process is activated during episodes but it is not clear why it is triggered.
What are the main symptoms of PFAPA syndrome?
The main symptoms of PFAPA are:
Episodic fevers
Sore throat
Mouth ulcers
Enlarged cervical lymph nodes (glands in the neck, an important part of the immune system)
The episodes of fever start abruptly and last for three to seven days. During episodes, the child looks very ill and complains about at least one of the three symptoms mentioned above. The episodes of fever recur every few weeks and often families know the exact day when an attack will start. On the day the fever starts the child will feel a little ill before the attack and the family knows an attack is about to start. Not all children have all symptoms, especially mouth sores. Some children have other symptoms, like:
Joint pain
Abdominal pain
Headache
Vomiting
Diarrhea
How is PFAPA syndrome diagnosed?
There are no laboratory tests, or imaging procedures, specific for diagnosing PFAPA. The disease will be diagnosed based on the results of a physical examination and other symptoms. Inflammatory blood tests like the white blood cell count, erythrocyte sedimentation rate and the C-reactive protein are increased during attacks. Before the diagnosis is confirmed, it is important to exclude all other diseases that may present with similar symptoms (especially a streptococcal throat). The dramatic response to treatment (see below) also helps diagnose PFAPA.
How is PFAPA syndrome treated?
There is no specific treatment to cure PFAPA. The aim of treatment is to control symptoms during the episodes of fever, to shorten the duration of episodes, and in some children to prevent attacks from occurring. In most children, the disease will resolve by itself without treatment, usually after the age of 10 years. The fever does not usually respond well to Tylenol®️ or nonsteroidal anti-inflammatory drugs. A single dose of steroids (usually prednisone), given when symptoms first appear, has been shown to shorten an episode and sometimes even end the episode. However, the interval between episodes may also be shortened with this treatment, and the next episode may occur earlier than expected. In some patients using cimetidine (a medicine that is used to treat stomach ulcers) may prevents attacks from occurring. In patients with very frequent attacks, a tonsillectomy (removing the tonsils by surgery) may be considered.
What is the outcome and course of PFAPA syndrome?
The disease may last for several years. Over time, the intervals between the episodes will increase and usually after the age of 10 years resolve by itself. Children with PFAPA continue to grow and develop normally."
— PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually *starts in early childhood between ages 2 and 5*. In very rare cases, the syndrome may start in adulthood.
DISCHARGE SUMMARY-
An 18 year old male who is a resident pf Hyderabad and a student presented with the chief complaints of fever since 4 days.
Vomiting since 4 days.
Generalised body pains since 4 days.
Cough without expectoration since 1 day.
History of presenting illness
The patient was apparently asymptomatic till the age of 5 years.
2008 (5years)
2/08/2008: diagnosed and treated for typhoid fever.
17/08/2008: He had high grade fever for two days. On the second day as the fever didn’t subside he was taken to the nursing home. While waiting for the doctor he had an episode of seizure. Due to this he was sent to a nearby hospital where he was treated for the same.
The seizure lasted for 5 mins. Drugs were given when taken to the hospital following which it subsided. Froth from the mouth was present. No history of bladder and bowel incontinence. Postictal drowsiness was present for 10-15 mins.
He was admitted in the hospital and discharged after 4 days.
Treatment given : Epsolin (phenytoin) it was continued for 1 week post discharge
No complaints for the next 3 months.
1/12/2008
Complains of cough, abdominal pain, watery discharge from nose
Sudden onset high grade fever with no chills and rigor since morning.
1 episode of seizure in the evening for 5 mins. Froth from the mouth was present. No history of bladder and bowel incontinence. Postictal drowsiness and confusion was absent.
Following which he admitted in the hospital.
He had another episode GTCS at 3am for 5 mins. Froth from the mouth was present. No history of bladder and bowel incontinence. Postictal drowsiness was absent. Tongue biting was observed.
CRP - raised
EEG - slow activity
Treatment given : epsolin (phenytoin), midazolam and diazepam for 5 days.
Patient was started on sodium valproate 2.5ml BD
2009 (6 years)
April 2009
Patient was advised to undergo adeniodectomy and tonsillectomy as it was a underlying cause for febrile seizures.
7 days post OP
1 episode of GCTS lasting 1 min followed by 40 mins of postictal drowsiness. Froth from the mouth was present. No history of bladder and bowel incontinence.
His CRP(1.2mg/dL) and ESR were raised.
Treatment: sodium valproate 3ml BD daily.
2010 (7 years)
June 2010
1 episode of GTCS lasting for 2 mins. Episode of vomiting during the seizure. Tongue biting was noticed. Froth from the mouth was present. No history of bladder and bowel incontinence.
CT brain - Normal
EEG- normal
Raised ALP: 708U/L
Other LFT were normal
Sodium valproate serum concentration was observed to be lesser than therapeutic level and thus it’s dosage was increased.
Treatment : sodium valproate 4ml BD daily
September 2010
Patient had abdominal pain for 3 days accompanied with episodes of vomitings and loose motions on the 3rd day
He was admitted in the hospital
CBP showed leukocutosis
LFT showed increased SGOT SGPT and ALP
Diagnosis given : Anti epileptic medication induced acute gastritis.
2012 (9 years)
April 2012
Patient had moderate to high grade fever since morning following which he had GTCS lasting for 2 mins. Froth from the mouth was present. No history of bladder and bowel incontinence. Tongue biting was seen.
He was admitted in the hospital for further evaluation
His serum sodium valproate concentration was noted to be above therapeutic range.
He was given Tegretol 7.5 ml BD daily and sodium valproate was stopped.
2013 (10 years)
July 2013
Abdominal pain for 10 days. On day 11th, 1 episode of GTCS lasting 2 mins
Froth from the mouth was present. No history of bladder and bowel incontinence. No tongue biting was seen.
EEG was normal
Tegretol was stopped
Frisium and Levipril were given for 2 weeks following which levipril was continued.
No episodes of seizures for 3 years
2016 (13 years)
July 2016
Patient had High grade Fever and multiple episodes of vomiting for 5 days.
He also had generalised body pain and neck stiffness
Clinical sings: nystagmus was present.
Could not walk in a straight line and could not perform Finger nose test.
CSF analysis : no abnormality
USG ABDOMEN AND PELVIS : B/L enlarged kidneys with grade 1 renal parenchymal changes. Transient proteinuria was present and was treated for the same.
2017 (14 years)
July 2017
High grade fever associated with chills and rigor. It was associated with neck pain which radiated to both upper limbs.
CSF analysis: no abnormality
MRI : normal
CRP : raised
TLC : raised.
Diagnosis: AFI with encephalitis
2018 (15years)
June 2018
High grade fever on and off since 1 week.
Cough, throat pain a generalised weakness since 1 week.
Admitted to hospital for further evaluation.
TLC : raised
CRP : raised
Proteinuria : 2+
Diagnosis given : AFI With acute bronchitis.
2019 (16 years)
6 years since the last episode of seizures.
April 2019
Levipril dose was tapered.
July 2019
Patient had high grade fever with chills and rigor. Diagnosed as AFI with sepsis.
During this fever episode, the dose of Levipril was increased as a prophylaxis to prevent the febrile seizures. It was tapered after the fever subsided.
August 2019
1 episode of GTCS lasting 2 mins. Froth from the mouth was present. Postictal confusion for 10 mins. No history of bladder and bowel incontinence. Tongue biting was seen. No history of vomiting.
LEVIPRIL dose was increased (500mg BD). It is being used till date.
2021(18 years)
February 2021
Increased frequency of micturition for 2 weeks (day time- 14-15 time night- 4-5 times) frothy urine was observed.
Cough and cold for 1 week. High grade fever for 5 days.
Diagnosed with UTI
Admitted in the hospital and treated for the same.
24 hour urinary protein was 2488mg/dL
CT abdomen : bulky bilateral renal parenchyma causing partial obliteration of renal sinus fat- likely early pyelonephritis
Patient was advised a renal biopsy which was not done.
After discharge the patient was still not relieved of the increased frequency of micturition.
April 2021
Ayurvedic medication started for increased frequency of micturition
May 2021
Normal frequency of micturition. Ayurvedic medication discontinued.
h/o rt ear discharge with pain(on and off) since 1 year. 2-3 episodes/yr lasting for 6-7 days. Last episode - 2months back.
Discharge- mucous, moderate, intermittent, non foul smelling, non blood stained. Treated for the same every time.
The patient was asymptomatic 4 days back when he developed high grade fever which was sudden in onset and varied between a range of 100-104 without touching the baseline. It was associated with chills and rigor
He has had episodes of vomiting on day 2 and day 4 of symptoms.
The vomitus contained food particles, it was non bilious and non projectile.
He has taken Ayurvedic medication on day 3 of fever for the same.
During the stay- had fever spikes(fever charting above); terminal rigidity and neck rigidity for 2 days; backache for 3 days
Abnormal- 24urinary protein value,RFT, leucocytosis, ESR(elevated). Reports above 👆
DIAGNOSIS-
Periodic fever since 5 yrs with periodic meningism and glomerular injury .
Treatment in his stay here-
Day 1-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
INJ.OPTINEURON 1AMP IN 100 ML NS
INJ.CEFTRIAXONE IG IV/BD
T.LEVIPIL 500MG PO/BD
TEMP. CHARTING
GRBS 12TH HRLY
Day2-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
INJ.OPTINEURON 1AMP IN 100 ML NS
INJ.CEFTRIAXONE IG IV/BD
T.LEVIPIL 500MG PO/BD
TEMP. CHARTING
GRBS 12TH HRLY
Day 3-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
INJ.CEFTRIAXONE IG IV/BD
T.LEVIPIL 500MG PO/BD
TEMP. CHARTING
GRBS 12TH HRLY
Day 4-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
Stopped antibiotics (ceftriaxone) in v/o negative blood culture and no infective foci
T.LEVIPIL 500MG PO/BD
TEMP. CHARTING
GRBS 12TH HRLY
Day 5-
IVF-NS,RL
PLENTY OF ORAL FLUIDS
T.DOLO 650MG SOS
Stopped antibiotics (ceftriaxone) in v/o negative blood culture and no infective foci
T.LEVIPIL 500MG PO/OD
TEMP. CHARTING
GRBS 12TH HRLY
Advice at discharge-
Tab. LEVIPIL 500mg OD x 1 week (to taper his dose)
Tab. Pantop 40mg po OD x 5 days
Tab. MVT PO OD x 5 days
REVIEW SOS.
POST DISCHARGE STATUS OF THE PATIENT-
The patient continues to have frothy urine with raised 24hr urinary protein levels.
The patients latest use abdomen shows a normal result in spite of a deranged CT done 10 months back (early pyelonephritis?).
His CUE
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